Cancer that begins in the kidney usually falls into one of these categories:
- Renal cell carcinoma. Overwhelmingly, this is the most common type of kidney cancer in adults, accounting for 90% of cases. It forms in the lining of the very small tubes in the kidney that filter the blood and remove waste products. Renal cell carcinoma has several subtypes, based on what the cells look like under a microscope:
- Clear cell renal carcinoma (most common)
- Papillary renal carcinoma
- Chromophobe renal carcinoma
- Non-clear renal cell carcinoma, including the very rare subtypes collecting duct and medullary
- Unclassified renal carcinoma
- Transitional cell carcinoma is far less common, only 5 to 10% of kidney cancers. Transitional cell carcinoma begins in the renal pelvis, the center of the kidney where urine collects.
- Wilms tumor is a very rare type of kidney cancer occurs in young children. Wilms tumor requires a different approach to treatment than adult kidney cancers and the specialized care of pediatric oncologists. Learn more about our pediatric cancer care.
- Renal sarcoma is a very rare cancer type, and less than 1% of kidney cancers. Renal sarcoma begins in the connective tissue or blood vessels of the kidney.
- Hereditary kidney cancer can occur in people who are born with mutations or alterations in specific genes, such as those seen in these genetic syndromes:
- Von Hippel-Lindau (VHL)
- Hereditary leiomyomatosis and renal cell carcinoma (HLRCC)
- Hereditary papillary renal cell carcinoma (HPRCC)
- Birt-Hogg Dubé (BHD)
Von Hippel-Lindau (VHL) syndrome and kidney cancer
A rare genetic disease, Von Hippel-Lindau (VHL) syndrome is caused by inheriting an abnormal VHL gene which can lead to multiple tumors or cysts of the eye, brain, pancreas, adrenal gland, kidney and other parts of the body. VHL is the most common cause of hereditary kidney cancer. If you or a relative have a known VHL gene mutation, talk to your doctor about tests you should have for early detection of kidney cancer and other cancers.
Kidney cancers in patients with VHL syndrome or other hereditary syndromes often need to be treated differently from regular kidney cancers and by a physician team with experience in recognizing and managing these syndromes. Roswell Park urologist Eric Kauffman, MD, serves on the VHL Alliance Clinical Advisory Council, an international committee that advises physicians on the specialty care required for patients with this hereditary syndrome.
Roswell Park is a designated VHL Clinical Care Center offering careful monitoring, early detection and timely treatment to reduce and prevent harmful effects of this and other hereditary kidney cancer gene mutations.